1. Expanding horizons: ciliary proteins reach beyond cilia.
- Author
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Yuan S and Sun Z
- Subjects
- Abnormalities, Multiple, Animals, Bardet-Biedl Syndrome genetics, Bardet-Biedl Syndrome physiopathology, Cell Movement, Cerebellar Diseases genetics, Cerebellar Diseases physiopathology, Cerebellum abnormalities, Cilia ultrastructure, Ciliary Motility Disorders genetics, Ciliary Motility Disorders physiopathology, DNA Damage, DNA Repair, Disease Models, Animal, Encephalocele genetics, Encephalocele physiopathology, Eye Abnormalities genetics, Eye Abnormalities physiopathology, Flagella physiology, Flagella ultrastructure, Heterotaxy Syndrome genetics, Heterotaxy Syndrome physiopathology, Homeostasis, Humans, Kidney Diseases, Cystic congenital, Kidney Diseases, Cystic genetics, Kidney Diseases, Cystic physiopathology, Molecular Motor Proteins physiology, Nervous System cytology, Polycystic Kidney Diseases genetics, Polycystic Kidney Diseases physiopathology, Polycystic Kidney, Autosomal Dominant genetics, Polycystic Kidney, Autosomal Dominant physiopathology, Polycystic Kidney, Autosomal Recessive genetics, Polycystic Kidney, Autosomal Recessive physiopathology, Retina abnormalities, Retina physiopathology, Retinitis Pigmentosa, Cilia physiology
- Abstract
Once obscure, the cilium has come into the spotlight during the past decade. It is now clear that aside from generating locomotion by motile cilia, both motile and immotile cilia serve as signaling platforms for the cell. Through both motility and sensory functions, cilia play critical roles in development, homeostasis, and disease. To date, the cilium proteome contains more than 1,000 different proteins, and human genetics is identifying new ciliopathy genes at an increasing pace. Although assigning a function to immotile cilia was a challenge not so long ago, the myriad of signaling pathways, proteins, and biological processes associated with the cilium have now created a new obstacle: how to distill all these interactions into specific themes and mechanisms that may explain how the organelle serves to maintain organism homeostasis. Here, we review the basics of cilia biology, novel functions associated with cilia, and recent advances in cilia genetics, and on the basis of this framework, we further discuss the meaning and significance of ciliary connections.
- Published
- 2013
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